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Lung involvement is the most frequent cause of death in patients with systemic sclerosis (SSc). As lung involvement is frequently asymptomatic, the current recommendation is to carry out thoracic computed tomography (CT) in all patients newly diagnosed with SSc. There is currently disagreement on how patients with SSc for whom no lung involvement was found at the time of diagnosis, should be followed up. Based on a consensus of Austrian rheumatologists, pneumologists and radiologists it is recommended that for asymptomatic patients with a negative CT at the time of initial diagnosis, a transthoracic ultrasound examination should be carried out annually and a lung function examination every 6-12 months. In the presence of a positive lung ultrasound finding a supplementary CT for further clarification is recommended. Based on the data situation, annual CT follow-up controls are recommended for patients with a high risk as defined by appropriate risk factors.Die Lungenbeteiligung ist die häufigste Todesursache bei Patienten mit systemischer Sklerose (SSc). Da eine Lungenbeteiligung häufig asymptomatisch ist, wird derzeit empfohlen, bei allen Patienten mit einer neu diagnostizierten SSc eine Thorax-CT durchzuführen. Uneinigkeit herrscht derzeit darüber, wie SSc-Patienten, bei denen zum Diagnosezeitpunkt keine Lungenbeteiligung gefunden wurde, weiterverfolgt werden sollen. Basierend auf einem Konsensus österreichischer Rheumatologen, Pneumologen und Radiologen, wird empfohlen, bei asymptomatischen PatientInnen mit einer negativen CT zum Zeitpunkt der Erstdiagnose jährlich transthorakale Ultraschalluntersuchungen sowie Lungenfunktionsuntersuchungen alle 6 bis 12 Monate durchzuführen. Bei Vorliegen eines positiven Lungenultraschallbefundes wird eine ergänzende CT zur weiterführenden Abklärung empfohlen. Aufgrund der Datenlage werden bei PatientInnen mit einem höheren Risiko, definiert durch entsprechende Risikofaktoren, jährliche CT-Verlaufskontrollen empfohlen.

Nailfold capillaroscopy is a rapid and easily applicable differential diagnostic technique that allows direct visualization of the microcirculation. Abnormal findings in nailfold capillaroscopy are closely associated with connective tissue diseases, such as systemic sclerosis. The clinical manifestation of impaired microcirculation is Raynaud's phenomenon, which is a classical symptom of connective tissue diseases. Nailfold capillaroscopy is increasingly used in various fields of medicine, therefore it is important to define methods for the acquisition and analysis of the results of nailfold capillary and to have a uniform definition of abnormal capillaries. This article discusses image acquisition and analysis, various capillaroscopic techniques, normal and abnormal capillaroscopic features and their significance, scoring systems and reliability of image acquisition and interpretation.Die Kapillarmikroskopie ist ein schnell und unkompliziert anwendbares differenzialdiagnostisches Tool, mit dem v. a. zwischen einem primären und sekundären Raynaud-Phänomen unterschieden werden kann. Bei verschiedenen Kollagenosen, vorrangig der systemischen Sklerose, ist sie sowohl zur Diagnostik als auch zur Aktivitäts- und Verlaufsbeurteilung unerlässlich. In der Kapillarmikroskopie feststellbare mikroangiopathische Muster lassen sich darüber hinaus auch bei weiteren Erkrankungen feststellen. In dieser Arbeit werden die Grundlagen der Untersuchung, deren Anwendung und klinische Relevanz dargestellt.

Systemic Sclerosis with Interstitial Lung Disease (SSc-ILD) is associated with an increased risk of morbidity and mortality. Because of a lack of approved medications that can effectively influence SSc-ILD disease course, there is a need for new therapeutic options. Treatment with immunomodulatory therapies as well as with autologous stem cell transplant is being further investigated in current clinical studies. Recently, a phase III study demonstrated the positive effect of the antifibrotic agent nintedanib on the loss of lung volume and thus disease progression in patients with SSc-ILD. Due to its synergistic mechanism of action, combination therapy with nintedanib and mycophenolate could be a complementary treatment approach for SSc-ILD in the future.Die systemische Sklerose mit interstitieller Lungenerkrankung (SSc-ILD) ist mit einem erhöhten Morbiditäts- und Mortalitätsrisiko verknüpft. Da es an zugelassenen Medikamenten mangelt, die den Krankheitsverlauf einer SSc-ILD effektiv beeinflussen, besteht ein hoher Bedarf an neuen Therapieoptionen. Die Behandlung mit immunmodulierenden Therapien sowie der autologen Stammzelltransplantation wird in klinischen Studien aktuell weiter untersucht. Kürzlich belegte zudem eine Phase-III-Studie die positive Wirkung des antifibrotischen Wirkstoffs Nintedanib auf den Verlust an Lungenvolumen und somit die Krankheitsprogression bei Patienten mit SSc-ILD. Eine Kombinationstherapie aus Nintedanib und Mycophenolat könnte basierend auf synergistischen Wirkprinzipien zukünftig einen ergänzenden Behandlungsansatz der SSc-ILD darstellen.

As part of the reappraisal of the legacy of Wismut AG, 12 patients with silica-induced scleroderma among underground uranium ore mine workers (Wismut AG) under long-term exposure to silica fine dust, as well as radon and its daughter products, during the 1960s and 1970s are reported on. Silica-induced scleroderma is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis. In experimental studies, endothelial cells, monocytes and fibroblasts, as well as their synthesis rates and the release of cytokines and chemokines, were activated by silica fine dust in a way that is consistent with the pathophysiological processes in idiopathic systemic sclerosis. It was not possible to achieve recognition of silica-induced systemic sclerosis as an occupational disease in Germany.Im Rahmen der Aufarbeitung des Wismut AG-Erbes wird über 12 Kranke mit quarzinduzierter Sklerodermie bei Beschäftigten im Uranerzbergbau (Wismut AG) unter Tage mit Langzeitexpositionen durch Quarzfeinstaub sowie Radon und seinen Folgeprodukten in den 60iger und 70iger Jahren des vergangenen Jahrhunderts berichtet. Die quarzinduzierte Sklerodermie kann aufgrund klinischer, serologischer und immunologischer Marker nicht von der idiopathischen Form unterschieden werden. In experimentellen Versuchen wurden Endothelzellen, Monozyten und Fibroblasten in ihren Syntheseleistungen und der Freisetzung von Chemo- und Zytokinen durch Quarzfeinstaub in einer Art und Weise aktiviert, die mit den pathophysiologischen Vorgängen bei idiopathischer Sklerodermie übereinstimmt. Die Anerkennung als Berufskrankheit konnte in Deutschland nicht erreicht werden.

In the last few years, several studies emerged for the treatment of interstitial lung disease (ILD) associated with systemic sclerosis (SSc). Some of the studied drugs showed efficacy compared to placebo or to a control group. Since early 2020, nintedanib has been approved for the treatment of SSc-ILD. Although several drugs are used to treat SSc-LD, it is now the first approved drug for SSc-ILD. Here, we aim to provide an overview about our current therapy algorithm and strategy as well as our interpretation of study results as an experienced SSc-ILD center.Zur Behandlung der interstitiellen Lungenerkrankung (ILD) bei systemischer Sklerose (SSc) wurden in den letzten Jahren verschiedene Studien durchgeführt. Einige zeigten eine Wirksamkeit in der Beeinflussung der SSc-ILD. Nintedanib ist im Frühjahr 2020 zur Behandlung der SSc-ILD zugelassen worden. Es ist somit das einzige bisher zugelassene Medikament für diese Indikation. In dieser Übersicht möchten wir als erfahrenes SSc-Zentrum unser therapeutisches Vorgehen aufzeigen und eine Einschätzung geben, wie die verschiedenen Therapieansätze zur Behandlung der SSc-ILD in unserem Zentrum eingeordnet werden und die Therapie aktuell erfolgt.

There have been three randomized controlled trials on autologous hematopoietic stem cell transplantation (AHSCT) in systemic sclerosis (SSc) that demonstrated significant superiority with respect to survival, improvement of cutaneous fibrosis, lung function and quality of life compared to standard treatment; however, these advantages must be carefully weighed against the transplantation-related risks. For this reason, an expert group from the stem cell therapy working party of the German Society for Rheumatology (DGRh) has now developed recommendations for the use of AHSCT in SSc. Based on the high-quality evidence, AHSCT is considered as the standard option for the treatment of selected SSc patients. Potential candidates for AHSCT are those with early, rapidly progressive, diffuse cutaneous SSc with visceral manifestations who have not yet developed severe damage to internal organs. A close cooperation between rheumatologists and transplantation centers is crucial for optimizing patient selection and treatment outcomes.

Scleroderma or systemic sclerosis (SSc) is a rare autoimmune rheumatic connective tissue disease. The clinical picture is manifold and symptoms can vary greatly between different patients. All manifestations are possible ranging from isolated skin involvement up to systemic disease with multiple organ manifestations. Due to this inhomogeneous clinical picture, it often takes years until the correct diagnosis is made and adequate treatment is started.Patients with the main or secondary diagnosis of systemic sclerosis (M34) between 2002 and 2017 were retrospectively recorded from the patient databases of the ACURA clinic for acute rheumatology in Bad Kreuznach and the data were evaluated. Of special interest were pulmonary parameters over the course of time. Furthermore, standardized questionnaires were distributed to general practitioners in Rhineland-Palatinate via the Association of Statutory Health Insurance Physicians as well as to patients admitted to the hospital (2016-2017).A total of 135 patients could be evaluated. For women the median age of onset was 52 years (interquartile range, IQR 44-64 years) and for men the median age of onset was 49 years (IQR 38-54 years). Lung involvement was detected in 54% of the cases. Including the individual time to diagnosis, there was a significant worsening of the diffusing capacity for carbon monoxide (73% vs. 56%, p = 0.046) between earlier (4 months) and later (4-18 months) diagnoses, which also persisted in the follow-up (74% vs. 53%) despite adequate treatment.A rapid diagnosis within 3 months of the onset of Raynaud's phenomenon seems to play a key role in the preservation of lung function.

Systemic sclerosis is a challenging and heterogeneous disease due to the involvement of multiple organs and the high impact on morbidity and quality of life. Lung fibrosis, pulmonary arterial hypertension, and cardiac manifestations are main causes of systemic sclerosis-related deaths. In addition, patients suffer from a various range of co-morbidities such as malnutrition, depression, osteoporosis, malignancies, which are increased in these patients and have to be identified and treated. Early assessment of organ damage is a key to therapeutic success. The discovery of pathogenic autoantibodies combined with increased evidence of effective immunosuppressive and vasoactive treatment strategies are major developments in the therapy of the disease. At present, several clinical studies are ongoing and some of the biological therapies are promising.

Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies.This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment".The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy.Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.

Raynaud's phenomenon (RP) is a frequent and painful vasospasm of small arteries localized in acral body regions (most frequently the fingers). The more frequent so-called primary RP is caused merely by a functional dysregulation of the tonus of vascular walls. In contrast, the rarer secondary RP is additionally associated with structural abnormalities of blood vessels. Knowledge of RP is important for rheumatologists because secondary RP can be associated with the presence or development of severe underlying diseases, especially with systemic sclerosis. Thus, the rheumatologist has to be aware of this condition. In this article the diagnostic procedures and the most important treatment approaches are summarized.

The skin is commonly affected in chronic inflammatory disorders and may act as a visual marker for internal or systemic inflammation. Frequent inflammatory skin diseases, like psoriasis and atopic dermatitis (AD), are associated with rheumatic and inflammatory bowel diseases. Metabolic, mental and cardiovascular comorbidity are frequent consequences of chronic inflammation. Further intersections between skin and joints are connective tissue diseases (collagenoses) and can be observed in complex diseases, e.g. systemic lupus erythematosus. Clinically, these diseases range from predominant cutaneous to severe systemic implication of several organs. Localized scleroderma should be clinically distinguished from systemic sclerosis and treated sufficiently to avoid long-term damage and disability. Thus, interdisciplinary disease management is of crucial importance.

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as a multiorgan disease must be distinguished from localized scleroderma or morphea because in addition to a different clinical picture they have a different prognosis and necessitate other therapeutic procedures. Rare sclerosing skin diseases with implications for internal medicine are eosinophilic fasciitis, Buschke's scleredema adultorum, scleromyxedema and nephrogenic systemic fibrosis.

Systemic sclerosis is a rare rheumatologic disease that is characterised by skin and organ fibrosis as well as vascular changes and the occurrence of specific autoantibodies. It has a high morbidity and mortality while its manifestations show significant heterogeneity in patients. Thus, diagnosis and follow-up of patients with systemic sclerosis has to be extensive, the more so because treatment must be adapted to organ manifestations. Although specific therapies for gastrointestinal, pulmonary or vascular complications exist, patients respond only partly to these and new therapeutic approaches are still needed.

This literature review summarizes the main findings in systemic sclerosis (SSc) made in the last few years. Accordingly, the disease pathogenesis is mainly driven by the adaptive immune system, which is proven by the effects of autologous stem cell transplantation. Particularly, autoantibodies can activate both adaptive as well as innate immune cells as identified for the anti-angiotensin receptor antibodies. In addition, major achievements come from the early recognition of organ complications, which mainly appear in the first years upon Raynaud`s phenomenon. This implicates screening for organ complications such as for pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD) even without any clinical symptoms at the beginning. On the other hand, the presence of anti-polymerase III antibodies indicates a risk or the presence of malignant diseases, which should be identified. Several studies in the last years showed the high burden of the disease, which is often underscored by physicians. Pain, depressions, fatigue, and incontinence often determine quality of life and should be recognized and treated, if possible. Systemic sclerosis is a disease with the highest disease-related mortality among the rheumatic diseases. More than half of the SSc patients die from SSc manifestations particularly from cardiac and lung involvement such as PAH and ILD. Ventricular tachycardias should be recognized by Holter-ECG. Finally, intensive therapies such as autologous stem cell transplantation or combination therapies seem to be most successful in SSc as well as in SSc-related PAH. Currently, several studies are ongoing, which will hopefully change the outcome and quality of life.Das adaptive Immunsystem scheint für die Pathogenese entscheidend. Insbesondere dürften Autoantikörper, die das angeborene und adaptive Immunsystem regulieren, eine wesentliche Rolle spielen. DAS FRüHE ERKENNEN VON ORGANKOMPLIKATIONEN: Schäden entstehen in den ersten 5 Jahren nach Beginn des Raynaud-Syndroms. Daher ist ein kontinuierliches Screening auf Organschäden nötig; die frühe Zuweisung in Zentren wird empfohlen. Bei Polymerase-III-Ak muss an ein paraneoplastisches Syndrom gedacht werden. Die kardiale Beteiligung ist prognostisch wichtiger als zuvor vermutet.Die Systemische Sklerose (SSc) besitzt eine Vielzahl von wenig bekannten Symptomen wie Inkontinenz, Juckreiz und beeinträchtigt in der Regel die Lebensqualität deutlich. DIE LETALITäT DER SYSTEMISCHEN SKLEROSE D: ie Mehrzahl der Patienten stirbt an der Erkrankung oder an deren Folgen, wobei neben der Lungenfibrose und der pulmonalen arteriellen Hypertonie die kardiale Beteiligung die knapp 10 %ige Sterblichkeit innerhalb von 2 Jahren verursacht. Durch frühes Erkennen von ventrikulären Tachykardien kann der plötzliche Herztod verhindert werden.Frühe und intensive Therapien und die Behandlung in Zentren sind nötig. Bei früher diffuser SSc ist eine autologe Stammzelltransplantation zu erwägen. Eine Reihe Substanzen befindet sich derzeit in Phase-III-Studien. Viele SSc-Patienten sind untertherapiert.

Raynaud's phenomenon (RP) is a painful vasospasm of small arteries, localised in fingers and toes. Typically these body parts turn white (ischemia), then blue (deoxygenation) and then red (reperfusion). Two different types of RP exist: the common primary RP without underlying disease and the rare secondary RP, mostly associated with rheumatoid diseases such as systemic sclerosis. Thus, the dermatologist has to be aware of this condition. In this article the clinical criteria, differential diagnoses, diagnostic considerations and treatment options are discussed.

The correct interpretation of skin manifestations can facilitate the diagnosis of many rare systemic diseases. Such manifestations can be due to autoimmune diseases (e.g. dermatomyositis, systemic lupus erythematosus, systemic sclerosis and sarcoidosis) and metabolic diseases (e.g. Anderson-Fabry disease and porphyria cutanea tarda). Other cutaneous symptoms are of great importance because they are possible warning signs of occult diseases of internal organs. This is true for example for some diseases from the group of neutrophilic dermatoses, such as Sweet's syndrome and pyoderma gangraenosum.

Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult.The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated.A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described.Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics.An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals.

Systemic sclerosis is a chronic connective tissue disease in which immune system dysfunction alters the structure and function of blood vessels. It is characterized by enhanced tissue fibrosis, which may rapidly affect a large area of the skin as well as internal organs. Dry eye symptoms are the most common ocular findings.This case presents a 51-year-old male patient suffering from blurred vision in the left eye. The ophthalmoscopic examination revealed retinal thrombotic microangiopathy in both eyes. Spectral-domain optical coherence tomography (SD OCT) showed a macular edema in the left eye. Although these ocular symptoms do not represent the initial manifestation of the primary disease, correct diagnosis was only made in the context of the retinal findings.Retinal microangiopathic lesions can play a seminal role in diagnosing systemic sclerosis.

Systemic sclerosis (scleroderma) is a severe, chronic inflammatory connective tissue disease involving the skin, musculoskeletal system, and several internal organs. The heterogeneity of its presentation and course are a particular challenge for the treating physician. As therapeutic options have improved considerably during recent years, various working groups have published consensus recommendations for the treatment of SSc. The aim of this overview is to present major aspects of these recommendations and embed them in a concept of modern interdisciplinary care for this often still devastating disease.

Pulmonary involvement in patients with underlying autoimmune diseases poses a major diagnostic and therapeutic challenge to the treating physician. Due to the associated increased mortality risk, early diagnosis is crucial.The incidence and mortality rate of connective tissue disease-related interstitial lung diseases (CTD-ILD) and pulmonary hypertension (PH) were evaluated in patients with rheumatic disease including clinical aspects, diagnostic procedure, prognosis and treatment recommendations.An analysis of remarkable publications was carried out and guidelines are presented.The CTD-ILD and PH are frequent comorbidities with significantly increased mortality risk, especially in patients with systemic sclerosis (SSc). In primary fibrotic and non-inflammatory CTD-ILD, as occurs especially in patients with rheumatoid arthritis, immunosuppressive therapy is only partially effective. Currently, in some eligible patients only lung transplantation remains as a definitive therapy.The diagnostics and treatment of CTD-ILD and PH in patients with an underlying autoimmune disease requires an interdisciplinary approach. The effectiveness of antifibrotic treatment needs to be evaluated in the future.

Pulmonary arterial hypertension (PAH) is a major complication of systemic sclerosis (SSc) and screening is recommended for a timely initiation of disease-targeted drug therapy to modify disease progression. Patients with SSc-PAH have a better prognosis when detected and treated early. The PAH can occur in all disease stages and subsets of SSc. Regular screening tests, which are indicative for PAH, e.g. echocardiography, diffusion capacity, brain natriuretic protein (BNP) and a 6-min walking test, are recommended to enhance the suspicion, since clinical symptoms are unspecific and occur late in the course of PAH. In patients with suspected PAH, the diagnosis should be confirmed by right heart catheterization. A multidisciplinary approach in expert centres including rheumatologists and respiratory physicians and cardiologists specialized in pulmonary hypertension is mandatory for management of patients with SSc at risk for or with manifest pulmonary arterial hypertension.

Our study compares the biomechanical properties of the cornea in patients with long-term pre-existing (for at least 10 years) systemic sclerosis (SSc) with those of healthy suspects.The examination was performed in 14 systemic SSc patients and 38 healthy volunteers. Non-invasive assessment of corneal biomechanical parameters, including central corneal thickness (CCT), corneal hysteresis (CH) and corneal resistance factor (CRF), was performed in one randomised study eye in accordance with a standardised protocol. Intraocular pressure (IOP) values were analysed using different measuring techniques, including Goldmann applanation tonometry (GAT), dynamic contour tonometry (DCT) and Ocular Response AnalyzerWhen measured with DCT and ORA-based NCT, IOP levels were significantly lower in SSc patients than in the control group (DCT IOP: p = 0.048, NCT IOPgat: p = 0.002, NCT IOPcc: p 0.001). CCT was also significantly reduced in the SSc (p = 0.001). There were no statistically significant differences between the groups in CH and CRF. The difference between the corrected values (CHcorr - CRFcorr) was negative in the two groups. This was slightly lower for the SSc patients (delta = - 0.83) than for the control group (delta = - 0.66). For SSc patients, there were highly significant negative correlations between CH and CRF and between CHcorr and CRFcorr. In contrast, in the control group there was a non-significant positive correlation between age and biomechanical properties.In the course of the disease SSc leads to various alterations in connective tissue, which can also involve corneal tissue. CCT becomes significantly thinner and simultaneously partially loses elastic properties and gains viscosity. This accounts for reduced IOP values with dynamic contour tonometry as well as with ORA-tonometry (Goldmann-correlated IOP, CCT-compensated IOP). This distinct pre-existing significant negative correlation between age and CH and CRF values in patients with SSc could be due to slight regression of the viscous components and diminution of corneal damping capacity.Die Studie vergleicht die biomechanischen Eigenschaften der Hornhaut bei Patienten mit einer seit mindestens 10 Jahren bestehenden systemischen Sklerodermie (SSc) mit einer gesunden Kontrollkohorte.Untersucht wurden 14 SSc-Patienten und 38 Kontrollprobanden. Ein randomisiertes Studienauge wurde auf die kornealen biomechanischen Eigenschaften zentrale Hornhautdicke (CCT), korneale Hysterese (CH) und kornealer Resistenzfaktor (CRF) untersucht. Die Bestimmung des Augeninnendrucks (IOP) erfolgte anhand der Goldmann-Applanationstonometrie (GAT) und der dynamischen Konturtonometrie (DCT) sowie mittels Non-Contact-Tonometrie (NCT) mit dem Ocular Response AnalyzerDer IOP war mittels DCT- und ORA-basierter NCT-Messung bei SSc-Patienten signifikant geringer als bei der Kontrollgruppe (DCT IOP: p = 0,048, NCT IOPgat: p = 0,002, NCT IOPcc: p = 0,000). Die CCT war ebenfalls bei SSc-Patienten signifikant dünner (p = 0,001). Bezüglich der CH und des CRF ergaben sich keine statistisch signifikanten Unterschiede. Die Differenz der korrigierten Parameter (CHZwischen den untersuchten Gruppen konnte keine Veränderung der biomechanischen Parameter (CH, CRF) festgestellt werden. Eine deutlich vorhandene signifikante negative Korrelation von Lebensalter und CH bzw. CRF bei SSc könnte durch eine leichte Degression der viskösen Komponente und Verringerung der kornealen Dämpfungskapazität bedingt sein. Die dünnere CCT und der erniedrigte Differenzwert lassen auf veränderliche Prozesse in der Zusammensetzung der kornealen Extrazellulärmatrix bei SSc-Patienten schließen.

The study aimed to evaluate the clinical feasibility of hybrid ultrasound/multispectral optoacoustic tomography (MSOT) for assessing microvascular dysfunction in systemic sclerosis (SSc). A handheld US/MSOT imaging system was applied for imaging patients diagnosed with SSc (n = 7) and healthy volunteers (n = 8). Semiquantitative MSOT values for deoxygenated (HbR), oxygenated (HbO(2)) and total haemoglobin (HbT) were analysed for subcutaneous finger tissue of both hands (8 fingers per subject, 120 fingers in total) and used to assess disease activity (progressive vs stable). Grouped data were compared by one-way nested analysis of variance, Tukey post-hoc test as well as student's t test were used for statistical analysis.Subcutaneous finger tissue of patients with SSc provided significantly lower MSOT values for HbO(2) (26.16 +/- 0.71 vs 38.2 +/- 1.54, P =.023) and HbT (55.92 +/- 1.62 vs 72.46 +/- 1.90, P =.018) compared to healthy volunteers. Patients with progressive SSc had significantly lower MSOT values compared to patients with stable disease and healthy volunteers.This pilot study shows the feasibility of MSOT imaging to resolve microvascular dysfunction in SSc as a marker of disease activity. By providing biological tissue properties not revealed by other imaging modalities, MSOT might help to grade SSc non-invasively and monitor early therapy response.

Systemic sclerosis (scleroderma) is a rheumatologic disease characterised not only by inflammation/autoimmunity, but also by tissue fibrosis and vascular lesions. The therapeutic approach to patients is dictated by the organ involvement and includes treatment of vascular and fibrotic disease features beyond mere immunosuppression. Fibrotic features in particular, are still inadequately treated, whereas many drugs have been tested for vascular complications within recent years. In this review, the currently available treatment options for this rare disease are presented. Therapy options in systemic sclerosis have changed over the past 10 years and this trend will also continue in the future.

Digital ulcers (DU) affect up to 60 % of patients with systemic sclerosis (SSc) and have a considerable impact on quality of life and morbidity. It is unclear to what extent authorised medicines are used, and if therapy guidelines are implemented in everyday practice.This retrospective health care study examined current standards of treatment for therapy and prevention of SSc-associated DU in an online survey with 83 physicians. Additionally, data from 161 case studies of SSc patients with DU were analysed, and the effect of DU treatment on the course of the disease determined.For treatment and prevention of active DU, physicians predominantly indicated topical therapies, calcium channel blockers, iloprost and endothelin receptor antagonists. According to the case studies, 90 % of episodes with acute DU were treated with bosentan and iloprost in mono- or combination therapy. Preventive treatment was only administered during 50 % of episodes without DU, even after three or more phases with active DU. For the prevention of new DU, bosentan was used in mono- or combination therapy in 57 % of episodes without DU. Bosentan therapy during prevention shortened the following acute phase by 32 %. Additionally, continuous treatment with bosentan in acute and prevention phases reduced the duration of the following acute phase and increased the time to onset of new DU by 16 %. Moreover, bosentan stabilised the number of new DU.In summary, these data confirm the efficacy of bosentan in preventing new DU when used in DU-free episodes and possibly also in phases of acute DU. Therapy recommendations for the treatment of DU are currently not fully implemented. In the future, even more attention should be paid to DU therapy.

Autologous hematopoietic stem cell transplantation (HSCT) is a very effective treatment option for patients with severe systemic sclerosis (SSc). In addition to various case series two randomized controlled trials could prove its superiority over intense cyclophosphamide pulse therapy. Nevertheless, HSCT is associated with a treatment-related mortality of approximately 10 %; therefore, further studies should be carried out to reduce the toxicity of HSCT by adaptation of the therapy regimen and the option of HSCT should be made available earlier to patients with a high risk of mortality. The mechanism of action of HSCT is still poorly understood. While profibrotic cytokines or even autoantibodies hardly appear to be influenced by the treatment, alterations to regulatory T‑cells may play a role. Further improvement of transplantation regimens as well as a better understanding of the underlying pathogenetic principles and mechanisms of action should be the aim of further studies on HSCT.

Systemic sclerosis (SSc) is a rare, chronic inflammatory autoimmune disease with unknown etiology, which leads to deposition of collagen and extracellular matrix proteins in the skin and affected internal organs. The diagnosis of SSc is based on clinical, serological, and paraclinical examinations. In 2013 new criteria for the classification of systemic sclerosis, which also take early forms of SSc into consideration, were developed. A complete clinical and paraclinical examination is important for the oligosymptomatic early stages and the subsequent disease course of SSc in order to diagnose and timely treat a developing organ involvement.

Large data bases and the projects arising from them have led to a much improved understanding of systemic sclerosis over the last decade. Serology has developed further so that more autoantibodies are available for routine testing. Capillary microscopy has become standard and relevant progress has also been made in therapy. Many diagnostic terms found in medical documentation do not adequately reflect this progress. The nomenclature is inconsistent and, therefore, confusing. The international classification of diseases (ICD) nomenclature is, from our point of view, also in need of improvement. This article aims to reestablish a common German language standard for systemic sclerosis, which reflects current knowledge and is suitable for implementation in the clinical routine.